BACKGROUND: Aggressive fibromatoses (desmoid tumours) may be locally aggressive, but do not metastasize. Although a conservative approach is advocated for most patients, pain and functional impairment are indications for active treatment. Tumour necrosis factor (TNF) α and melphalan-based isolated limb perfusion (TM-ILP) is a limb-saving treatment modality for soft tissue tumours. This study reports the results of TM-ILP treatment in patients with aggressive fibromatosis. METHODS: Institutional databases of three European centres were searched...

Acetaminophen or paracetamol, a commonly used over-the-counter analgesic, is known to elicit severe adverse reactions when taken in overdose, chronically at therapeutic dosage or, sporadically, following single assumptions of a therapeutic dose. Damage patterns including liver damage and, rarely, acute tubular necrosis or a fixed drug exanthema. We present a case of fatal acetaminophen toxicity with postmortem blood concentration 78 μg/mL and unusual clinical features, including a visually striking and massive epidermolysis and rhabdomyolysis, disseminated intravascular coagulation and myocardial ischemia...

OBJECTIVE: Subepidermal bullous lesions and toxic epidermal necrolysis-like (TEN-like) lesions can occur in patients with systemic lupus erythematosus (SLE). In this report, we describe a case of a patient previously diagnosed with SLE who experienced TEN-like skin lesions with unusual subacute progression in the context of the current literature. METHODS: We present a recent case of TEN-like lupus erythematosus and review of studies published in English identifying SLE cases mimicking TEN, accessed via PubMed and Google Scholar databases...

A 58-year-old female with a history of dermatomyositis was receiving large oral doses of steroids. She had pulmonary tuberculosis and developed a fever, systemic exudative erythema, exanthema, and epidermolysis covering 30% of her body surface area while being treated with four agents, including isoniazid (INH) and rifampicin (RFP). Histopathologically, eosinophilic necrosis was observed in all layers of the epidermis and a diagnosis of Stevens-Johnson syndrome (SJS) progressive toxic epidermal necrolysis (TEN) was made...

Clinical symptoms of toxic epidermal necrosis (TEN) include massive epidermolysis within skin and mucous membranes. Similar to extensive stage II burns, this may be a life-threatening condition. Patients with TEN are usually treated in specialized burn centers andlor surgery departments. Here we present and discuss 2 case reports of patients with ocular complications of TEN. The aim of this study is to emphasize the necessity of ophthalmic control and treatment during the early and late phase of TEN.

HISTORY AND CLINICAL FINDINGS: A 34-year-old woman was admitted for treatment of toxic epidermolysis of the skin and mucosa. 16 days previously she had started to take chlormezanone (Muskel Trancopal) and some other medications for pain in the shoulder and neck. On admission she had a fever of 39 degrees C and, in addition to the epidermolysis, diffuse abdominal pain on pressure and blood-streaked stool. INVESTIGATIONS: Liver enzyme activities (GOT 979 U/I, GPT 1496 U/I, gamma GT 201 U/I) alkaline phosphatase 515 U/I), bilirubin (3...

Toxic epidermal necrolysis is one of the rarest events -- a dermatologic emergency. The clinical presentation is characteristic -- disseminate erythema and widespread bullous necrosis of the epidermis and mucous membranes. Toxicity is universal and mortality is substantial even with prompt and intensive therapy. Drugs are the most commonly incriminated agents and therapy remains controversial, the majority favoring high-dose systemic corticosteroids.

Staphylococcal toxic shock syndrome (TSS) and staphylococcal scalded skin syndrome (SSSS) are two distinct toxin-mediated syndromes with prominent cutaneous features. The exanthematous presentation of these syndromes places them in the broad category of childhood exanthems, and the ability to recognize these potentially devastating illnesses is essential for pediatricians and dermatologists who may encounter children with fever and rash. Recent advances in the understanding of the pathogenesis of these entities has helped to explain the distinctive clinical presentations of TSS and SSSS...

The scalded skin syndrome or Lyell syndrome can now be divided into two distinct forms. One form is associated with staphylococci that are usually phage group II Staphylococcus aureus, are penicillin resistant, and produce a protein exotoxin which produces epidermolysis by causing a split to develop in the epidermis between the stratum granulosum and the stratum spinosum. The other form is associated usually with drug reactions and is characterized by necrosis of the epidermis with a split between epidermis and dermis...

Acute epidermal necrosis is an exceptionally serious condition whose onset, which is sometimes preceded by a few prodomes, is marked by an eruption mainly around the orifices and rapidly extending to the whole of the tegument. Epidermal necrosis results in extensive shedding of the skin and within the space of a few hours gives the patient the appearance of having been scalded. In adults, drugs and in particular certain combinations of drugs, are responsible; the drugs mainly involved are the sulfonamides, especially delayed action sulfonamides, derivatives of butazolidine, Barbiturates, Phenindione and Penicillin...